Abstract

OBJECTIVE: Intracranial ependymoma is a rare brain tumor in adults. The standard treatments comprise of aggressive resection and radiotherapy, although only a few contemporary studies have evaluated their prognostic values. The objective of this study is to analyze clinical data from Brain Tumor Registry of Japan for assessing prognostic factors in adult intracranial ependymoma. MATERIALS AND METHODS: Seventy-six adult patients of intracranial ependymoma treated at 31 hospitals between 2001 and 2004 were included in the study. Subependymoma was excluded. Clinical data collected prospectively were retrospectively analyzed. RESULTS: The median age was 45 years (range,18-85), and the median KPS score was 80 (range,40-90). Infratentorial and multiple tumors were noted in 40 and 4 cases, respectively. Forty-five cases were classified as WHO grade 2 and 29 cases as grade 3. Grade 3 tumors were significantly associated with low KPS score, supratentorial tumor, large tumor diameter, and seizure onset compared to grade 2 tumors. All the grade 3 tumors except one were treated with radiotherapy and/or chemotherapy, while only 18 were treated with radiotherapy among 45 grade 2 tumors. With a median follow-up of 40 months, 5-year overall survival (OS) was 98% for grade 2 and 46% for grade 3; the median OS was not reached for grade 2 and 44 months for grade 3. Five-year progression-free survival (PFS) was 85% for grade 2 and 33% for grade 3; the median PFS was not reached for grade 2 and 21 months for grade 3. Multivariate analyses revealed that WHO grade (P = 0.009), complete resection (P = 0.002), and KPS score (P = 0.02) were associated with OS and that WHO grade (P = 0.02) and complete resection (P = 0.003) with PFS. CONCLUSION: WHO grade 3 ependymoma more often presents as a supratentorial mass provoking seizure than grade 2. WHO grade and complete resection were independent prognostic factors in adult intracranial ependymoma.

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